Open AccessRenal Epithelioid Angiomyolipoma Associated with Pulmonary Lymphangioleiomyomatosis: Imaging Findings
Author(s) -
Athina C. Tsili,
Alexandra Ntorkou,
Maria I. Argyropoulou
Publication year - 2017
Publication title -
journal of clinical imaging science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.279
H-Index - 18
eISSN - 2156-7514
pISSN - 2156-5597
DOI - 10.4103/jcis.jcis_14_17
Subject(s) - tuberous sclerosis , medicine , lymphangioleiomyomatosis , angiomyolipoma , pathology , magnetic resonance imaging , lung , computed tomographic , radiology , kidney , computed tomography
Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are the most common tumors of the perivascular epithelioid cell (PEComa) family. Both may be associated with tuberous sclerosis (TS) complex. Epithelioid AML (EAML) is a rare variety of AMLs, with a potential aggressive behavior. There are few reports in the English literature addressing on the imaging findings of renal EAMLs, which are considered nonspecific. We present the sonographic, computed tomographic, and magnetic resonance imaging findings of a renal EAML in a pregnant woman with concomitant pulmonary lesions indicative of LAM, without stigmata of TS. We conclude the importance of considering EAML as a possible diagnosis in the presence of a large renal mass with high cellular content and small amounts of fat in the coexistence of pulmonary LAM